Flashbacks

Gosh, it has been a crazy couple of weeks. Where to start…

BLUF:

Joey is still in the PICU, and remains critical with acute liver failure. Again. His kidneys have recovered well. He is still intubated. His abdominal fluid build up has subsided, but he has immense swelling. While we still are waiting for an official diagnosis, he is being treated for an overactive immune condition, which can wreak havoc on the liver. He can’t be re-transplanted until this immune condition is under control, or it will attack his new liver (like it appears to be attacking his current liver). The treatment could heal his liver entirely, but only time will tell. Until then, the doctors are doing all they can to sustain his current liver. We hope to know by the end of the weekend/early next week if this treatment is working, and whether his liver is recoverable.

More details:

What we thought was going to be a quick trip to get Joey’s drain replaced on the 10th has snowballed into another liver failure nightmare. After Joey had his drain repositioned in the morning, the interventional radiology doctor took a look at it’s position via x-ray in the afternoon and found that it had moved out of its intended place. Joey was to go back down to have the drain fixed, again, and another one added to his left side (the first drain was on his right side). This would ensure coverage of his whole abdomen, or as much as possible. The interventional radiologists had other emergency surgeries to complete, so they wouldn’t get back to Joey until later in the evening. In the meantime, Joey went for a CT scan to get a better sense of how much fluid there was in his abdomen, and if there were any pockets of fluid. When he returned from his CT scan, Joey went into respiratory failure. After being intubated earlier in the day, the tube was giving him all sorts of issues, and the placement was very finicky. From transferring him around to get the CT scan, it must have jostled something around. The medical team spent a while trying to get it positioned just right when he came back to his room. After a while, they decided to just put in a new tube. While transitioning to a new tube, he was provided oxygen via a bag. But each time they tried to put in the new tube, they couldn’t get it in within the 20-30 seconds they had with the bagged oxygen. We were told this was likely because of all of the fluid build up, pushing on his diaphragm and lungs. 

Chris and I stood outside of his room, calmly at first, giving the respiratory team and attending some space while they were adjusting Joey’s tube. All of the sudden, people were running in and out of his room, people were yelling, we heard the monitoring beeping like crazy. There were suddenly many more people, including anesthesiologists. I swear there were probably 15 people in there at one point. We became aware very quickly that something wasn’t going well. Things became pretty blurry after that, but a nurse came out and told us that they had the right people in the room, and all of the medications needed in case things took a turn, but that it could go either way. We waited outside of his room, for what felt like eternity, holding each other tight and praying hard that Joey would pull through. Thank God he did. The pediatric anesthesiologist was able to finally get the tube in, but in the process Joey’s oxygen level plummeted (anything below 90% can be considered dangerous. Joe’s level dropped to 9%), as did his heart rate. He was given Epinephrine and Atropine to help boost him back. Luckily those worked quickly and he didn’t need chest compressions.

It is hard to even think about that evening. We knew the transplant surgery was risky, but to be right outside of the room (but not allowed in), listening to the chaos of your child almost losing their life was much different. I have never felt more helpless and scared. It was the worst night of my life.

Overall though, Joey’s lungs were great. It was decided to keep him intubated to reduce the stress on his lungs of breathing with all of the fluid built up pressing against everything. Eventually some of the fluid leaked in between the tissue of his lungs and chest as well. And, as the week went on, he became extremely swollen. The fluid build up had become so bad, that it just finally started leaking into his soft tissue. He looked like a yellow Michelin man (his skin is yellow again from elevated bilirubin levels). As these kept piling on throughout the week, it was decided to keep him intubated, again, to reduce stress on his lungs from the fluid and additional weight he had gained (the doctor said it was the equivalent of an adult gaining about 30lbs).

Throughout the week, his liver numbers remained somewhat stable. They weren’t in range, but they weren’t necessarily getting worse. We felt hopeful about this, thinking his liver was on the road to recovery. And would have a real shot if we could just address the fluid issues. The medical team continued doing their best to address the fluid input/output, but when certain numbers in his labs dipped too low, he’d need some blood products (red blood cells, platelets, etc.), which kept his fluid balance on the positive side. Not great when you are trying to get rid of fluid, but again, it is such a delicate balance. It got to the point where they were trying to further concentrate his medications, and add some to his TPN (IV nutrition), just to minimize the fluid he needed just to survive.

By Friday, he was looking better. His stomach was flat for the first time in a while, but he was still a little puffy. We all felt happy with the progress on the fluid front, and were hopeful that with a lot of it gone, his liver would have some room to breathe and recover.

Saturday morning, the 16th, Joey had a seizure. Chris had just gotten to the hospital, and we were working our way out of the room to meet the team for daily rounds. The nurses had just been adjusting Joey’s position, turning him to his side to keep him moving, and he roused from sedation and was a little agitated (normal for him). Chris went to say hi to him, and the nurses were helping settle him a bit. So I walked out of the room. The nurses followed shortly after. After 30 seconds or so, Chris hadn’t come out. I went back in the room to see what was going on, and Chris said he was trying to get Joey to calm down. I told him I would trade, and he could go out for the rounds. But when I looked down at Joey, he didn’t look just agitated. Chris and I have never seen anyone have a seizure before, but from enough tv, I had a feeling that was one. I ran out to the team and called them in. They quickly gave him a dose of Fentanyl and Ativan. He stopped seizing almost immediately after.

The next two days were filled with an ultrasound, CT scan, EEG test, and MRI to find out if he had a stroke, had bleeding in his brain (part of the liver’s function is to help with blood clotting… so when your liver isn’t working, you are at a higher risk for bleeding), whether there was swelling and permanent damage, whether he was likely to have more, etc. Luckily, everything came back negative. But of course, like everything else going on with Joey, we don’t really have concrete answers about why that happened.

The seizure felt like a setback. With the different testing, we couldn’t stay on top of his fluids as well, so he started to puff back up. From the CT and MRI, they also found an obstruction in his bowel, making his intestines swell. So, during one of his tests, they put in a large sump NG tube that sucks fluid and air out of his stomach. That seemed to help quite a bit, and looks okay for now. But we are still monitoring that issue.

During these few days, his fluid stopped accumulating, but his liver numbers started getting worse, as did his white blood cell count and platelets. The Transplant Attending brought up a possible diagnosis of HLH, an inflammatory condition. This is essentially when your immune system goes into overdrive and attacks good cells in your body, instead of an infection or virus (don’t quote me, I’m still learning this stuff). Primary HLH is genetic. Secondary HLH is usually triggered by something like an infection (or a major organ transplant, or possibly even COVID… which Joey had both of in January).

HLH was brought up in January as a potential explanation for Joey’s acute liver failure then. But to have an official diagnosis of HLH, you need to meet five of eight criteria. In January, Joey only met one or two of the criteria. It was too risky to biopsy his liver then to see if he had HLH, or something like it, present in those cells, but the team sent out genetic testing. However, due to the fact that it is sent to a third party lab somewhere else in the country, and genetic sequencing takes a while, we just got those results back on Wednesday (he was negative for HLH, so Primary HLH has been ruled out). We of course didn’t have time to wait for those results then, and the team made the decision to list him then with what information they had.

Now, Joey still only meets four of the criteria for HLH. But the reason the doctors think Joey might have this, or something similar, is because it is quite literally the only thing that can explain everything that is going on with him now (between his liver failure and blood issues). And they are thinking that post-transplant, all of his immunosuppressants held off this inflammatory response for a little while, so it is just starting to hit full force.

HLH is typically treated with high-dose steroids, chemotherapy, antibody medications, and potentially, a stem cell or bone marrow transplant. This essentially suppresses his immune system to allow it to reboot, or with stem cells/bone marrow, replace his cells (again, don’t quote me… still trying to learn this). So, we have been speaking with the Hematology/Oncology team about this diagnosis and treatment. Additional tests, to include a bone marrow biopsy, should be able to tell us if he has Secondary HLH, or something similar. Even though we don’t have all testing back to confirm a true HLH diagnosis, and he doesn’t meet enough criteria, we have moved forward with the steroids and antibody medications for now. The Hem/Oc team told us to think of it as a sliding scale, so even though it might not be a formal HLH diagnosis, it is so similar it would require the same type of treatment. They are calling it mini HLH for now.

So where does that leave his liver? We don’t know, unfortunately. Theoretically, HLH treatment could restore his liver. But because it took a hit from the excessive fluid too, it could be damaged beyond repair, even if the HLH treatment works to cure his immune system. Wednesday, Joey was taken back to the OR so the surgeons could take a look at his liver and surgically biopsy it. We should have those results by the weekend, and will have a better sense of the recoverability of his liver, and whether they see HLH present. The surgeon said his liver looked inflamed (not surprising), but it was smooth (so no excessive scarring present). He said it looked like it could be recovered. To give his liver some space to breathe while the inflammation dies down, they added a vertical incision (so now Joey has the true Mercedes incision one typically gets with a liver transplant), and left a wound vac (essentially mesh covering the opening) instead of closing him up. They’ll continue to reassess the state of his liver and likely slowly close him back up over the next week or so.

Even if his liver is damaged beyond repair, we can’t relist him for transplant in this condition. If he were to get a new liver, his immune system would likely attack it right away, if he even made it off of the operating table. So he isn’t a candidate for a new liver, should he need one, until we can get his immune system in a steadier state. Donor organs are a finite, precious resource, and shouldn’t go to waste. It is a harsh reality for me right now, but one I can understand. I wouldn’t want Joey to miss out on an organ that went to someone whose body wasn’t truly able to receive it.

So, we are waiting to see if this HLH treatment will work and how his liver responds, and trying to sustain him until we know. He is getting daily plasmapheresis treatments again to help maintain his liver. So far this seems to be keeping him steady, if not helping him ever so slightly improve his liver and blood numbers. We should know more by the end of the weekend/early next week if he is taking to the HLH treatment so far. Depending on how it is going, they may add in chemo or something similar (he currently can’t get the chemo treatment because of his liver failure). Once we have the liver biopsy results back, we should have a better sense of how long his liver has.

The timing is so tight. It feels like January all over again. I’m scared we won’t have enough information when we need to make some really tough choices. I’m scared we won’t even have choices if we can’t get his immune system addressed in time. But I’m clinging hard on to the surgeon saying the liver looked like it could be recovered. It gives me hope that his liver at least has some time while we sort out the immune system problems.

Thank you to those who have reached out to check in, sent words of love and encouragement, prayed for our baby boy, and us. We are so grateful for all of the support and love we continue to feel from everyone. We never thought we would be back in this situation. But we got through it once, and with all of you by our sides, we can do it again.

Please continue to hold Joey close in your thoughts and prayers. We will continue to make sure he feels all of the love, strength, and warmth from us, and all of you.

All our love,

The Moschellas